Pediatric Cardiology
Pediatr Cardiol 13:125-126, 1992
9 Springer-Verlag New York lnc, 1992
Cor Triatriatum Associated with Total Anomalous Pulmonary Venous D r a i n a g e in t h e S e t t i n g o f Mitral A t r e s i a a n d a R e s t r i c t i v e lnteratrial Communication Fadel AI-Fadley, Omar Galal, Neil Wilson, and Saud Atoufi Department of Cardiovascular Diseases, King Faisal Specialist Hospital, Riyadh, Saudi Arabia
SUMMARY. A rare variant of cor triatriatum is described in which the proximal "accessory" chamber received all four pulmonary veins and drained into the ievoatriocardinal vein and then into the innominate vein. The distal "true" chamber contained the left atrial appendage. The left atrioventricular valve was atretic and the interatriai septum was restrictive. KEY WORDS: Cor triatriatum--Total anomalous pulmonary venous drainage--Mitral atresia
In most cases of cor triatriatum the proximal chamber of the left atrium receives; all the pulmonary veins, but it has also been found in association with various forms of partial anomalous pulmonary venous connections. To our knowledge, there has been only one previous report of cor triatriatum associated with total anomalous pulmonary venous connection in which the mitral valve was normal and there was a large interatrial communication [5].
Case Report A 2-week-old male infant was born after normal gestation and delivery. Tachypnea and cyanosis were noted on the first day of life, and the baby was transferred to our hospital for further management. Physical examination revealed a normal-appearing baby: respiratory rate was 70/rain and heart rate 150 beats/min with normal peripheral pulses. Blood pressure was 85/60 mmHg. The precordial examination revealed a left parasternal heave. On auscultation the first heart sound was normal, but the second heart sound was single. There was a grade 2/6 soft ejection systolic murmur heard over the left lower sternal border. The liver was 3 cm below the right costal margin. Twelve-lead electrocardiogram revealed a sinus rhythm, normal QRS axis, and a QR pattern in vl suggestive of right ventricular hypertrophy. Chest x-ray study revealed cardiomegaly and pulmonary venous congestion. Echocardiography indicated a normal atrial situs, levocardia, and a left-sided aortic arch. The atrioventricular connection was concordant with an
Address offprint requests to: Dr. Fadel AI-Fadley, Department of Cardiovascular Diseases, P.O. Box 58460, Riyadh 11594, Saudi Arabia.
atretic left atrioventricular valve. The left ventricle was hypoplastic and the right ventricle connected with both great arteries, which were normally related. The pulmonary venous return was not well delineated. There was a small persistent foramen ovale. Cardiac catheterization was performed to evaluate further the pulmonary venous drainage and to allow for possible ballon atrial septostomy. Complete right-sided blood oxygen saturations and hemodynamic profiles were obtained in room air. Angiography was performed in the main pulmonary artery, but findings were not conclusive. A second angiogram was performed in the left atrium in the anteroposterior and lateral projections. In the early phase of the injection (Figs. 1A and 2A), the distal "true" left atrium was opacified and was in continuity with the left atrial appendage. In the later phase, there was opacification of the proximal "posterior" left atrial chamber to which all four pulmonary veins connected. Opacified blood in the proximal chamber subsequently drained through a left atriocardinal vein to the innominate vein and finally to the right-sided superior vena cava (Figs. 1B and 2B). There was a good-sized communication between the proximal and distal chambers of the left atrium. Because there was no hemodynamic or angiographic evidence of obstruction of the pulmonary venous drainage balloon atrial septostomy was not performed. The patient was discharged home on digoxin and furosemide, with a systemic saturation of 85%.
Discussion The embryogenesis of cor triatriatum remains a matter of controversy. Loeffler [2] suggested that failed incorporation of the common pulmonary veins into the left atrium was the basis of the lesion. Van Praagh and Corsini [4] proposed the entrapment theory that, during fetal life, the common pulmonary vein is trapped between the left horn of the
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Fig. 1. Left atrial angiogram in the anteroposterior projection. (A) In the early phase of the injection there is opacification of the distal left atrial chamber (DLA); (B) subsequent opacification of the proximal LA chamber (PLA), pulmonary veins, and levoatriocardinal vein.
Fig, 2. Lateral projection of the same injection as in Fig. 1. (A) Early frame showing the distal left atrium (DLA); (B) a later frame showing all pulmonary veins connected to the proximal left atrium (PLA).
sinus venosus and the sinus venosus itself. Since the embryological origin of the lungs is from the foregut, the pulmonary veins are connected with the systemic venous system during fetal life. Failed development of the common pulmonary vein results in total anomalous pulmonary venous connection and, therefore, persistence and growth of the pulmonary to systemic venous communications to form alternative pathways for pulmonary venous drainage. The simultaneous occurrence of total anomalous venous drainage and cor triatriatum is difficult to explain using either theory. Our case of cor triatriatum, total anomalous venous drainage, mitral atresia, and restrictive interatrial communications is atypical in several ways. It appears to be only the second report of total anomalous venous drainage associated with cor triatriatum. However, our case differs from the previously reported ones in that there was also mitral atresia and a restrictive atrial septum. Although all pulmonary veins were connected to the left atrium, an anomalous channel, "the levoatriocardinal vein," provided an alternative pathway from the left atrium to the innominate vein, which allowed for nonobstructed pulmonary venous drainage [3].
Our case demonstrates the difference between total anomalous pulmonary venous drainage and connection, terms that are often used synonymously. In our case there was a normal pulmonary venous connection but abnormal pulmonary venous drainage [1]. References 1. Delisle G, Andro M, Galder AI, et al. (1976) Total anomalous pulmonary venous connection: Report of 93 autopsied cases with emphasis on diagnosis and surgical consideration. A m Heart J 91:99-122 2. Loeffler E (1949) Unusual malformation of the left atrium pulmonary sinus. Arch Patho148:371-376 3. Lucas RV Jr, Lester RG, Lillehei CW, Edwards JE (1962) Mitral atresia with levoatriocardinal vein, a form of congenital pulmonary venous obstruction. Am J Cardiol 9:607 4. Van Praagh R, Corsini I (1969) Cor triatriatum: Pathologic anatomy and consideration of morphogenesis based on 13 postmortem cases and a study of normal development of the pulmonary vein and atrial septum in 83 human embryos. A m Heart J 78:379-405 5. Vouche PR, Baillot-Vernant F, Fermont L, Leca F, Neveux JY (1985) Cor triatriatum and total anomalous pulmonary venous connection; a rare surgically correctable anomaly. J Thorac Cardiovasc Surg 90:443-445
Pediatr Cardiol 13:125-126, 1992
9 Springer-Verlag New York lnc, 1992
Cor Triatriatum Associated with Total Anomalous Pulmonary Venous D r a i n a g e in t h e S e t t i n g o f Mitral A t r e s i a a n d a R e s t r i c t i v e lnteratrial Communication Fadel AI-Fadley, Omar Galal, Neil Wilson, and Saud Atoufi Department of Cardiovascular Diseases, King Faisal Specialist Hospital, Riyadh, Saudi Arabia
SUMMARY. A rare variant of cor triatriatum is described in which the proximal "accessory" chamber received all four pulmonary veins and drained into the ievoatriocardinal vein and then into the innominate vein. The distal "true" chamber contained the left atrial appendage. The left atrioventricular valve was atretic and the interatriai septum was restrictive. KEY WORDS: Cor triatriatum--Total anomalous pulmonary venous drainage--Mitral atresia
In most cases of cor triatriatum the proximal chamber of the left atrium receives; all the pulmonary veins, but it has also been found in association with various forms of partial anomalous pulmonary venous connections. To our knowledge, there has been only one previous report of cor triatriatum associated with total anomalous pulmonary venous connection in which the mitral valve was normal and there was a large interatrial communication [5].
Case Report A 2-week-old male infant was born after normal gestation and delivery. Tachypnea and cyanosis were noted on the first day of life, and the baby was transferred to our hospital for further management. Physical examination revealed a normal-appearing baby: respiratory rate was 70/rain and heart rate 150 beats/min with normal peripheral pulses. Blood pressure was 85/60 mmHg. The precordial examination revealed a left parasternal heave. On auscultation the first heart sound was normal, but the second heart sound was single. There was a grade 2/6 soft ejection systolic murmur heard over the left lower sternal border. The liver was 3 cm below the right costal margin. Twelve-lead electrocardiogram revealed a sinus rhythm, normal QRS axis, and a QR pattern in vl suggestive of right ventricular hypertrophy. Chest x-ray study revealed cardiomegaly and pulmonary venous congestion. Echocardiography indicated a normal atrial situs, levocardia, and a left-sided aortic arch. The atrioventricular connection was concordant with an
Address offprint requests to: Dr. Fadel AI-Fadley, Department of Cardiovascular Diseases, P.O. Box 58460, Riyadh 11594, Saudi Arabia.
atretic left atrioventricular valve. The left ventricle was hypoplastic and the right ventricle connected with both great arteries, which were normally related. The pulmonary venous return was not well delineated. There was a small persistent foramen ovale. Cardiac catheterization was performed to evaluate further the pulmonary venous drainage and to allow for possible ballon atrial septostomy. Complete right-sided blood oxygen saturations and hemodynamic profiles were obtained in room air. Angiography was performed in the main pulmonary artery, but findings were not conclusive. A second angiogram was performed in the left atrium in the anteroposterior and lateral projections. In the early phase of the injection (Figs. 1A and 2A), the distal "true" left atrium was opacified and was in continuity with the left atrial appendage. In the later phase, there was opacification of the proximal "posterior" left atrial chamber to which all four pulmonary veins connected. Opacified blood in the proximal chamber subsequently drained through a left atriocardinal vein to the innominate vein and finally to the right-sided superior vena cava (Figs. 1B and 2B). There was a good-sized communication between the proximal and distal chambers of the left atrium. Because there was no hemodynamic or angiographic evidence of obstruction of the pulmonary venous drainage balloon atrial septostomy was not performed. The patient was discharged home on digoxin and furosemide, with a systemic saturation of 85%.
Discussion The embryogenesis of cor triatriatum remains a matter of controversy. Loeffler [2] suggested that failed incorporation of the common pulmonary veins into the left atrium was the basis of the lesion. Van Praagh and Corsini [4] proposed the entrapment theory that, during fetal life, the common pulmonary vein is trapped between the left horn of the
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Pediatric Cardiology Vol. 13, No. 2, 1992
Fig. 1. Left atrial angiogram in the anteroposterior projection. (A) In the early phase of the injection there is opacification of the distal left atrial chamber (DLA); (B) subsequent opacification of the proximal LA chamber (PLA), pulmonary veins, and levoatriocardinal vein.
Fig, 2. Lateral projection of the same injection as in Fig. 1. (A) Early frame showing the distal left atrium (DLA); (B) a later frame showing all pulmonary veins connected to the proximal left atrium (PLA).
sinus venosus and the sinus venosus itself. Since the embryological origin of the lungs is from the foregut, the pulmonary veins are connected with the systemic venous system during fetal life. Failed development of the common pulmonary vein results in total anomalous pulmonary venous connection and, therefore, persistence and growth of the pulmonary to systemic venous communications to form alternative pathways for pulmonary venous drainage. The simultaneous occurrence of total anomalous venous drainage and cor triatriatum is difficult to explain using either theory. Our case of cor triatriatum, total anomalous venous drainage, mitral atresia, and restrictive interatrial communications is atypical in several ways. It appears to be only the second report of total anomalous venous drainage associated with cor triatriatum. However, our case differs from the previously reported ones in that there was also mitral atresia and a restrictive atrial septum. Although all pulmonary veins were connected to the left atrium, an anomalous channel, "the levoatriocardinal vein," provided an alternative pathway from the left atrium to the innominate vein, which allowed for nonobstructed pulmonary venous drainage [3].
Our case demonstrates the difference between total anomalous pulmonary venous drainage and connection, terms that are often used synonymously. In our case there was a normal pulmonary venous connection but abnormal pulmonary venous drainage [1]. References 1. Delisle G, Andro M, Galder AI, et al. (1976) Total anomalous pulmonary venous connection: Report of 93 autopsied cases with emphasis on diagnosis and surgical consideration. A m Heart J 91:99-122 2. Loeffler E (1949) Unusual malformation of the left atrium pulmonary sinus. Arch Patho148:371-376 3. Lucas RV Jr, Lester RG, Lillehei CW, Edwards JE (1962) Mitral atresia with levoatriocardinal vein, a form of congenital pulmonary venous obstruction. Am J Cardiol 9:607 4. Van Praagh R, Corsini I (1969) Cor triatriatum: Pathologic anatomy and consideration of morphogenesis based on 13 postmortem cases and a study of normal development of the pulmonary vein and atrial septum in 83 human embryos. A m Heart J 78:379-405 5. Vouche PR, Baillot-Vernant F, Fermont L, Leca F, Neveux JY (1985) Cor triatriatum and total anomalous pulmonary venous connection; a rare surgically correctable anomaly. J Thorac Cardiovasc Surg 90:443-445
